Comorbidity of behavioral problems and parental acceptance-rejection in children diagnosed with chest discomfort, palpitations, vasovagal syncope, and underlying heart disease: a multiple case-control study.

BACKGROUND: Children who experience chest discomfort, palpitations, vasovagal syncope, and underlying heart disease often present a complex clinical picture. Not only are they dealing with potential cardiac issues, but they may also exhibit behavioral problems that can complicate the diagnostic and treatment process. Moreover, parental acceptance or rejection can significantly influence the child's well-being and medical outcomes in such cases. This study aims to explore the comorbidity of behavioral problems and parental acceptance-rejection in children diagnosed with these cardiac symptoms and underlying heart disease. METHODS: In a case-control study, the Parental Acceptance - Rejection Questionnaire and Parental version of Strengths and Difficulties Questionnaire (SDQ) was filled by parents of 314 patients from pediatric cardiology clinic. RESULTS: The control group scored substantially lower overall according to SDQ. The vasovagal syncope subgroup was found to have considerably lower scores on the subscale. The group with chest discomfort scored highly in hostility and aggression in the PARQ. In comparison to the other groups, the vasovagal syncope and chest pain group demonstrated higher scores in undifferentiated rejection and total score. CONCLUSION: This study showed a correlation between children's behavioral and emotional problems and cardiac symptoms. This states that children's relationship with their parents has an impact on their symptoms. It will be necessary to conduct further studies to determine a causal association and devise preventative measures.

Behavioural interventions to increase adherence to palivizumab prophylaxis in children with CHD.

OBJECTIVES: Adherence to palivizumab prophylaxis programmes is crucial to protect infants with CHD against respiratory syncytial virus infections. We analysed the effectiveness of two nudge interventions in increasing adherence. METHODS: Our study included 229 infants, and their caregivers, from five centers in Turkey in the 2020-2021 respiratory syncytial virus season. We randomly allocated caregivers to a control and two intervention groups. Caregivers in all groups were informed about the prophylaxis programme and provided a schedule. Additionally, caregivers in Intervention 1 were called two days before appointments (default bias) and were asked to plan the appointment day (implementation intention), whereas caregivers in Intervention 2 received biweekly text messages informing them about the programme's benefits (availability bias) and current adherence rate (social norm). RESULTS: Caregivers in Intervention 1 had a significantly higher adherence rate than Control (97.3% versus 90.9%) (p = 0.014). Both interventions had a significant effect on participants in their first prophylaxis season (p = 0.031, p = 0.037). Families where the father was employed had a 14.2% higher adherence rate (p = 0.001). Every additional child was associated with a 2.2% decrease in adherence rate (p = 0.02). In control, ICU admission history was associated with an 18.8% lower adherence rate (p = 0.0001), but this association disappeared in intervention groups. CONCLUSION: This is the first prospective interventional study which, in the context of palivizumab prophylaxis, analyses the effectiveness of nudge interventions based on established cognitive biases by comparing randomly generated intervention and control groups. We found that default bias and implementation intention have significant effects on adherence.Clinical trial, in the name and number "Adherence of palivizumab prophylaxis, NCT05778240" registered retrospectively. https://clinicaltrials.gov/ct2/show/NCT05778240.

Effects of levetiracetam treatment on autonomic nervous system functions in pediatric epilepsy patients.

BACKGROUND: This study investigated the effects of levetiracetam (LEV) treatment on cardiac rhythm and heart rate variability. METHODS: The study included two groups of patients diagnosed with non-lesional epilepsy who had not yet been treated and who presented to the outpatient pediatric neurology clinic at Van Training and Research Hospital, Van, Turkey, between 2019 and 2020. The heart rate variability (HRV) of 47 patients in the first group, before and at the 3rd month of treatment, and intravenous (IV) LEV loading in 13 patients in the second group was evaluated by Holter electrocardiography (ECG). RESULTS: It was determined that the values of triangular index, standard deviation of the RR intervals over a 24-hour period (SDNN), standard deviation of all 5-minute mean RR intervals (SDANN), mean of standard deviations of all normal RR intervals (SDNNI), the percentage of RR intervals with >50-millisecond variation (PNN50), and the square root of mean squared differences of successive RR intervals (RMSSD). HRV of 47 patients under LEV treatment significantly increased in the 3rd month of treatment compared to baseline (p < 0.05). No difference was found in HRV between the intravenous loading and the control group (p > 0.05). CONCLUSIONS: Our study suggests that the sympathovagal balance before treatment in the patient group is in favor of the sympathetic nervous system and that the sympathovagal imbalance improves after treatment. Our results show that LEV monotherapy and loading have no negative effect on HRV and potential cardiac arrhythmia risk in children with epilepsy.

A Challenging Interventional Procedure: Transcatheter Closure of Tubular Patent Ductus Arteriosus in Patients with Pulmonary Hypertension.

Transcatheter closure of the tubular ducts remains the most challenging procedure, with higher complication rates than other types. This study evaluates the characteristics of transcatheter closure of tubular ducts with pulmonary hypertension. 73 patients with tubular ducts who underwent cardiac catheterization for transcatheter PDA closure were analyzed. The mean age and weight were 1.93 ± 2.68 years and 8.83 ± 6.14 kg, respectively. Transcatheter closure was attempted in 72 patients. Four cases (5.5%) were referred to surgery, while the procedure was completed in the remaining (94.5%). Amplatzer duct occluder (ADO) I or Cardiofix duct occluder (CDO) was the most commonly used devices. However, the use of Amplatzer vascular plug (AVP) II raised in recent years. The most common concern was aortic protrusion/stenosis in ADO I/CDO devices, but most regressed during follow-up. Iatrogenic coarctation of the aorta was observed in two with ADO I/CDO. Embolization of the device to the pulmonary artery was observed in three with CDO, AVP II, and AVP I. Significant left pulmonary artery stenosis requiring stenting developed in one after closure with an MVSDO device. Tubular ducts are highly associated with pulmonary arterial hypertension, and transcatheter closure of them is still challenging despite the developing device armamentarium. Although ADO I or similar devices are widely used, off-label devices are usually needed at increasing rates. The AVP II device is unsuitable for short tubular ducts but seems the best option for long ones.

Cross or Not to Cross-Dilemma of the Pulmonary Arteries.

We aimed to assess the frequency of criss-cross pulmonary arteries and associated intracardiac and vascular anomalies in patients who underwent CT angiography due to suspected congenital heart disease or vascular anomaly at our hospital. We retrospectively evaluated the CT angiography images of 355 patients aged 0-18 years between April 2018 and December 2022. The presence of the criss-cross pulmonary artery anomaly was assessed. Additionally, in patients with a criss-cross pulmonary artery anomaly, accompanying branch pulmonary artery anomalies, aortic arch anomalies, and other vascular-cardiac anomalies were also evaluated. A total of 331 patients' images were evaluated. Criss-cross pulmonary artery anomaly was present in 57 patients (17.2%). Pulmonary artery branch anomaly was present in 16, aortic arch anomaly in 40 patients (70%) with criss-cross pulmonary artery anomaly, while associated intracardiac pathology (by echocardiography) was detected in 43 patients (75.4%). The frequency of criss-cross pulmonary artery was found to be significantly higher in patients with any aortic arch anomaly (p = 0.01). This study represents one of the largest series of patients with criss-cross pulmonary artery anomalies. Our results suggests that it may be more common than previously recognized and potentially overlooked. It is crucial to consider the presence of this anomaly in patients with complex aortic arch anomalies or cardiac pathologies, as it may have implications for surgical approaches and potential complications. Increased awareness of this anomaly among cardiologists and radiologists is necessary for accurate diagnosis and appropriate management.

Increased serum concentrations of growth differentiation factor-15 in children with acute rheumatic fever.

AIM: In this single-centre prospective study, we aimed to evaluate the role of growth differentiation factor-15 in children with acute rheumatic fever. METHODS: The study group included 25 children with acute rheumatic fever, and the control group included 25 healthy children. In addition to routine laboratory tests used in the diagnosis and treatment of acute rheumatic fever, growth differentiation factor-15 levels of the study group (at the time of diagnosis and after the treatment) and the control group were assessed and compared. RESULTS: The mean growth differentiation factor-15 level of the study group at the time of diagnosis (918.40 ± 605.65 pg/ml) was significantly higher than the mean post-treatment level (653.08 ± 330.92 pg/ml) (p = 0.015). Similarly, the mean growth differentiation factor-15 level of the study group at the time of diagnosis was significantly higher than the control group (p = 0.04). However, mean growth differentiation factor-15 levels were similar between the groups after the treatment. Growth differentiation factor-15 was positively correlated with both C-reactive protein (p < 0.001) and erythrocyte sedimentation rate (p = 0.001) at the time of diagnosis. CONCLUSION: Growth differentiation factor-15 levels are significantly increased in children with acute rheumatic fever at the time of diagnosis and return to similar levels with healthy children after treatment. Growth differentiation factor-15 is positively and significantly correlated with erythrocyte sedimentation rate and C-reactive protein at the time of diagnosis.

A rare cause of hydrops fetalis in two Gaucher disease type 2 patients with a novel mutation.

Gaucher disease type 2 is the most progressive and the rarest form of Gaucher disease, defined as the acute neuronopathic type. We presented two GD2 patients who died before three months of age due to severe septicemia, respiratory and liver failure. One was homozygous for a novel GBA variant c.590 T > A (p.197 K), and the second homozygous for the known GBA mutation c.1505G > A (p.R502H). Ichthyosis, hydrops fetalis, apnea, myoclonic seizures, and hepatosplenomegaly occurred in both patients, but hypertrophic cardiomyopathy was observed only in the second and unilateral cataract in the first patient. Due to the disease's early and rapid neurological progression, we did not administer ERT to our patients. It is strongly believed that early diagnosis is essential, and prenatal diagnosis makes genetic counselling possible for future pregnancies.

Acute myocarditis following COVID-19 mRNA vaccination: a paediatric case.

Myocarditis is an inflammation of the heart muscle. In this case, a previously healthy, 17-year-old adolescent with myocarditis after BNT162b2 mRNA vaccination was reported. He was admitted to the hospital with severe chest pain, changes in electrocardiography, and elevation in serum troponin level after fourth day of receiving first dose of vaccine. There was no coronary arterial disease in coronary angiogram. A diagnosis of vaccine-induced myocarditis was made, and supportive treatment was initiated.

Congenital biventricular cardiac diverticula as a part of class III Cantrell's syndrome: case report.

Congenital cardiac ventricular diverticulum is an extremely rare condition that usually occurs as a part of Pentalogy of Cantrell and frequently associated with sternal, pericardial, diaphragmatic, and thoracoabdominal wall defects. The prognosis of the patient depends on the complexity of abnormalities. Herein, we report biventricular diverticula as a part of incomplete Cantrell's syndrome in a 1-day-old newborn.

Cardiovascular anomalies in Seckel syndrome: report of two patients and review of the literature.

Seckel syndrome is a very rare autosomal recessive disorder also known as bird headed dwarfism". It is characterised by proportional short stature, low birth weight, dysmorphic facial appearance, and mental retardation. In addition to its dysmorphic features, skeletal, endocrine, gastrointestinal, haematologic, genitourinary, and nervous system has been involved. Cardiovascular features very rarely associate with Seckel syndrome. We report two patients with Seckel syndrome, one with dilated cardiomyopathy and the other with multiple ventricular septal defects. Dilated cardiomyopathy and isolated ventricular septal defect have not been previously reported in Seckel syndrome. Cardiovascular evaluation should be performed in all patients with Seckel syndrome. Early diagnosis of congenital and acquired heart diseases will reduce morbidity and mortality in these patients.

Efficacy of levetiracetam in the treatment of Sydenham chorea.

BACKGROUND: To study the effect of levetiracetam in treating Sydenham chorea. METHODS: We retrospectively collected the data of 140 patients diagnosed with Sydenham chorea in the pediatric neurology and pediatric cardiology outpatient clinics of Van Training and Research Hospital between January 2010 and December 2018. RESULTS: There were 140 patients, 102 (70%) of whom were girls, with mean age of onset 11.8 ± 2.7 years. Symptomatic treatment was initiated in all patients at the time of diagnosis; this medication was changed during follow up in 15 patients. The most frequently prescribed drugs were haloperidol and sodium (Na) valproate, and the most frequently discontinued one was haloperidol, due to side effects. The second-choice drug was most often levetiracetam. Clinical response often began within the first 2 weeks, with Na valproate (P = 0.002), within 4 weeks with carbamazepine (P = 0.037) but 1-6 months with haloperidol (P = 0.018) and levetiracetam (P = 0.008). Time to full remission was similar with Na valproate, carbamazepine, haloperidol, and levetiracetam (P = 0.276). Our study indicated that levetiracetam was as effective as the other commonly used drugs in the symptomatic treatment of Sydenham chorea. CONCLUSION: Levetiracetam might be an option in the treatment of Sydenham chorea because of its acceptable effect and safety profile. This observation needs further support with evidence obtained through controlled and blinded trials.

Transcatheter radiofrequency ablation using near-zero fluoroscopy in children with fascicular ventricular tachycardia: a single-centre experience.

OBJECTIVE: Fascicular tachycardia is a common form of sustained idiopathic left ventricular tachycardia. This study aimed to achieve successful results with catheter ablation procedures performed through three-dimensional electroanatomic mapping using near-zero fluoroscopy in fascicular tachycardia patients. METHODS AND RESULTS: In this study, we included 33 consecutive children with fascicular tachycardia, for whom we performed a transcatheter radiofrequency ablation procedure using the EnSite® system. Activation mapping was performed in all patients during tachycardia, and the earliest presystolic purkinje potentials were the target site for radiofrequency lesions. RESULTS: Twenty-five patients were male, and eight were female. The mean weight of the patients was 39.6 ± 10.4 kg, and the mean age was 13.6 ± 2.5 years. The mean procedure time was 121.3 ± 44.3 minutes. The mean follow-up period was 18.4 ± 6.5 months. No fluoroscopy was needed in 30 patients. The mean fluoroscopy time in the remaining patients was 166.6 ± 80 seconds. All of the patients had left posterior fascicular tachycardia except for one who had left anterior fascicular tachycardia. The acute success rate was perfect (100%). No patients developed left bundle branch block or complete atrioventricular block. Recurrence developed in one patient. CONCLUSION: We suggest that radiofrequency ablations via an electroanatomic mapping system are quite safe and effective, with high success rates in paediatric patients with fascicular tachycardia. This method has the advantage of avoiding ionising radiation exposure for both the patient and operator, thus reducing the lifetime risk of malignancy in the paediatric population.

Diastolic dysfunction in patients with brucellosis despite the absence of infective endocarditis.

BACKGROUND: Brucellosis is an important systemic infectious disease, especially in developing countries. Every organ and system of the human body can be affected; however, cardiovascular complications of brucellosis are rare. AIM: To assess cardiac functions in patients with acute brucellosis without overt cardiac involvement and to answer the following question: Is there any cardiac dysfunction despite the absence of endocarditis in these patients? METHODS: This cross-sectional study included 67 children with brucellosis and 40 healthy children. We performed a detailed echocardiographic examination in individuals at the beginning of the treatment. Patients with infective endocarditis were excluded from the study. RESULTS: Echocardiography revealed no difference of ejection fraction, mitral and tricuspid annular plane systolic excursion, pulsed-wave Doppler-derived early diastolic peak velocity (E)/late diastolic peak velocity (A) ratios in mitral and tricuspid valves between the two groups. The deceleration time of early mitral inflow was longer in patients with brucellosis. Early diastolic peak velocity of the mitral and tricuspid annuluses obtained by tissue Doppler imaging (Ea) was significantly lower in children with brucellosis. The peak velocity obtained by tissue Doppler imaging during late diastole (Aa), Ea and Ea/Aa ratios in the interventricular septum, left ventricle posterior wall and right ventricle free wall was lower in patients with brucellosis than in the control group. The E/Ea ratio, isovolumic relaxation time, right ventricle and left ventricle myocardial performance indices were higher in patients with brucellosis. CONCLUSION: Patients with acute brucellosis may have diastolic dysfunction without overt cardiac involvement and infective endocarditis.

A rare coincidence: the long QT syndrome and cardio-facio-cutaneous syndrome.

Cardio-facio-cutaneous syndrome is a genetic anomaly characterised by craniofacial dysmorphia, developmental retardation, skin lesions, mental retardation/learning disability, and cardiac malformations. Cardio-facio-cutaneous syndrome rarely causes arrhythmias and has not been previously associated with long QT in the literature. With this report, it was aimed to draw attention to a different presentation of the long QT syndrome.

Occurrence of nutritional hypocalcaemic rickets-related dilated cardiomyopathy in a child with concomitant rickets and infantile-onset Pompe disease.

Infantile-onset Pompe disease is a lysosomal storage disorder characterised with hypertrophic cardiomyopathy, respiratory insufficiency, and hypotonia. Dilated cardiomyopathy is an extremely rare and curable complication of nutritional hypocalcaemic rickets. A 3-month-old female infant was referred to our paediatric ICU with a 4-day history of fatigue, tachypnoea, tachycardia, hypoxia, and respiratory failure. According to the laboratory, radiology, and echocardiography findings, she was first diagnosed with nutritional hypocalcaemic rickets-related dilated cardiomyopathy, but vitamin D and elementary calcium supplementation unmasked the underlying infantile-onset Pompe disease. Nutritional hypocalcaemic rickets and infantile-onset Pompe disease must always be kept in mind among the causes of concomitant dilated cardiomyopathy and hypertrophic cardiomyopathy.

Acute rheumatic fever presenting with severe endocarditis involving four valves, and ventricular tachycardia.

Acute rheumatic fever is the most commonly acquired heart disease in developing countries. The most common cardiac presentation is valvular disease. Although some rhythm disturbances may occur during the acute stages of the disease, ventricular tachycardia is extremely rare. Here, a case of acute rheumatic fever with severe endocarditis involving four valves and ventricular tachycardia is presented.

Anaphylactic reaction to anakinra in a child with steroid-dependent idiopathic recurrent pericarditis and successful management with canakinumab.

Conventional algorithm for treatment of pericarditis and prevention of recurrences consists of non-steroid anti-inflammatory drugs and/or colchicine, followed by corticosteroids in resistant patients. Anakinra has emerged as a promising and safe treatment modality for steroid-dependent idiopathic recurrent pericarditis. However, the efficacy and safety of canakinumab, another anti-interleukin-1 agent, has not been assessed up to date. Herein, we present development of an anaphylactic reaction due to anakinra and a successful subsequent treatment with canakinumab for the first time in the literature.

A beneficial technique for preventing the device protrusion to the aorta during percutaneous patent ductus arteriosus closure: "Balloon-assisted device releasing technique".

BACKGROUND: Although percutaneous closure of patent ductus arteriosus is an established safe procedure, protrusion of the device to descending aorta may occur in various degrees during these procedures, especially in small infants. The aim of our study is to evaluate the benefits of balloon-assisted device releasing technique in the era of preventing device protrusion and conditions related to protrusion. METHODS: One hundred and fifty-five infants, who underwent patent ductus arteriosus closure with Amplatzer duct occluder I device between January, 2012 and December, 2018, were retrospectively analysed. Balloon-assisted device releasing technique was used in 20 cases (group 1, 12.9%), between January, 2015 and December, 2018. Procedures in which the technique had been used were compared with the remaining ones (group 2, 87.1%, n = 135) with regard to device stabilisation, aortic disc protrusion to the aorta, iatrogenic coarctation, and device embolisation. RESULTS: There was no significant difference by means of gender, age, weight, and the ductal diameter, whereas the average mean pulmonary artery pressure was significantly higher in group 1. Device protrusion and related complications were significantly higher in group 2; thus, additional catheterisations or surgical interventions were required, while no additional intervention was required in group 1. CONCLUSION: The balloon-assisted device releasing technique provides a good device stabilisation and prevents protrusion of the device and related complications during percutaneous patent ductus arteriosus closure in selected cases.

Preliminary results of the CeraflexTM PDA occluder and device behaviour during releasing.

INTRODUCTION: The CeraFlexTM PDA occluder is a new flexible device with a unique delivery system that may be beneficial with regard to not changing the device position after releasing. We prospectively evaluate the efficacy of the device and also the device behaviour patterns during release. METHODS: The study included 21 patients. Their median age was 1.2 years (from 6 months to 28 years) and weight was 9.6 kg (from 5.4 to 82 kg). All of the ducts were conical except one atypical ductus. Median ductal diameter at the pulmonary end was 3.8 mm (from 2.2 to 8.2 mm). The ductus was closed using an antegrade approach, but special attention was paid to the patterns of device behaviour during and just after releasing. RESULTS: Three different modes of device behaviour were observed during and just after releasing: (1) Neither difficulty nor change of position in 13 patients (62%), (2) a little difficulty in releasing but no change of position in 6 (29%), and (3) change of the device position in 2 (9%). There was no residual shunt on the next day except in one patient, in whom late device embolisation occurred. The device was retrieved and another, bigger device implanted. CONCLUSION: The CeraFlexTM PDA occlude device seems to be safe and efficacious for patent ductus arteriosus closure. Its unique delivery system generally fixes the device in a stable position that does not change after release (91%). Minor difficulty in releasing is not uncommon; however, the major disadvantage is the need for larger sheaths for delivery.